Dermatológia pre prax 3/2024

Hay-Wells syndrome – a case report

Purpose: Hay-Wells syndrome, is a rare autosomal dominant disease which belongs to the ectodermal dysplasias. Case report: Authors presents the case of a 28-year-old man who was repeatedly hospitalized at the Dermatovenerology Department in theyears 2014, 2019 and 2022. The patient has continued to develop morbid skin manifestations in the area of the scalp since childhood with the appearance of cicatricial alopecia. The patient was first hospitalized in the dermatology department in the spring of 2014, when the condition was diagnosed as Pseudo kerion Celsi. In the patient, the diagnosis of Hay-Wells syndrome was highly probable due to the characteristic facial dysmorphia and associated diseases. Conclusion: Skin lesions in the scalp may be the first visible manifestation of systemic diseases. The clinical manifestations of alopecia are variable, and some diseases may produce clinical and histopathological features similar to this diagnosis. Awareness of this condition is important in the evaluation of biopsy in correlation with clinical manifestations which are helpful in early diagnosis and proper patient management.

Keywords: Hay-Wells syndrome, ectodermal dysplasias, cicatricial alopecia